Summary: The giant congenital melanocytic nevus is a rare entity with an extremely low incidence that appears at the time of birth and generally involves the dermis but may also affect other skin layers. According to its clinical evolution, the probability of malignancy may vary, so proper follow-up is essential for potential management. There is no consensus in the literature about the greater benefit of surgical versus nonsurgical management. In this case report, we present the surgical management of a school-aged patient using dermal substitutes and skin grafts, subjectively obtaining an improvement in his quality of life.